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MRI of autosomal dominant pure spastic paraplegia
K Krabbe1, J E Nielsen, E Fallentin
1Danish Research Center of Magnetic Resonance, Hvidovre Hospital, Hvidovre, Denmark.
Abstract:
We examined 16 patients with autosomal dominant pure spastic paraplegia (HSP) and 15 normal controls matched for age and sex using MRI of the brain and spinal cord. Images were assessed qualitatively by two independent radiologists, blinded to the clinical diagnosis. Areas of the brain and corpus callosum on one midsagittal slice and the area of the brain on one axial slice were measured and a "corpus-callosum index" expressing the size of the corpus callosum relative to that of the brain was calculated. Cross-sectional areas and anteroposterior and transverse diameters of the spinal cord at the levels of C 2, C 5, T 3, T 6, T 9 and T 11 were measured. No significant differences between patients and controls were found on qualitative evaluation of the images. The patients had a significantly smaller corpus callosum and "corpus-callosum index" than controls. This finding, not reported previously, might indicate that the disease process in pure HSP is not confined to the spinal cord. The anteroposterior diameters of the spinal cord at T 3 and T 9 were significantly smaller in patients than in controls. This might correspond to the degeneration of the pyramidal tracts and the dorsal columns described at neuropathological examination.
Insights
Autosomal dominant pure spastic paraplegia (HSP) patients show smaller corpus callosum size and reduced spinal cord diameters compared to controls. These findings suggest the disease may affect brain structures beyond the spinal cord.
Area of Science:
- Neurology
- Neuroimaging
- Genetics
Background:
- Autosomal dominant pure spastic paraplegia (HSP) is a neurological disorder primarily affecting the spinal cord.
- The full extent of brain involvement in pure HSP remains incompletely understood.
Purpose of the Study:
- To investigate potential brain and spinal cord structural differences in patients with autosomal dominant pure spastic paraplegia (HSP) compared to healthy controls using MRI.
- To explore whether the disease process in pure HSP extends beyond the spinal cord.
Main Methods:
- MRI scans of the brain and spinal cord were acquired from 16 patients with pure HSP and 15 age- and sex-matched controls.
- Qualitative image assessment by blinded radiologists.
- Quantitative measurements of brain areas, corpus callosum size, corpus-callosum index, and spinal cord dimensions at multiple levels.
Main Results:
- No significant qualitative differences were observed between patients and controls.
- Patients with pure HSP exhibited a significantly smaller corpus callosum and corpus-callosum index compared to controls.
- Significantly reduced anteroposterior spinal cord diameters were found in patients at the T3 and T9 levels.
Conclusions:
- The observed reduction in corpus callosum size suggests that the disease process in pure HSP may involve brain structures, not solely the spinal cord.
- Smaller spinal cord diameters at specific levels correlate with neuropathological findings of pyramidal tract and dorsal column degeneration.
- These neuroimaging findings provide novel insights into the potential central nervous system involvement in autosomal dominant pure spastic paraplegia.